Lymphangiomas are malformation of lymphatic system. Rare entity. 4% of all vascular tumors. Mostly benign.

1. A Case Report
Presented By :
Dr. Kamal Kant Gupta
Department of Surgery
SMS Medical College, Jaipur

2. • Lymphangiomas are malformation of lymphatic
system.
• Rare entity.
• 4% of all vascular tumors.
• Mostly benign.
• Can occur at any age and any part of the body, but
90% occurs in children <2 yrs age and involves
head and neck.

3. Blind sac theory:
Lack of lymphatic connection
Lymphatic Proliferation and dilation
a. May be either congenital or acquired.
b. Congenital-Often Associated with chromosomal
abnormalities.
c. Acquired- Trauma, inflammation, lymphatic obstruction.
d. Associated Factors- Maternal alcohol use, viral infection
during pregnancy.

4. • Histologicaly-
1.Capillary
2. Cavernous
3. Cystic Hygromas
4. Hemangiolymphangioma
• Depending on the size of cysts-
1. Micro cystic
2. Macro cystic
3. Mixed

5. • Depending on location & extent of disease-
I. U/L IH
II. U/L SH
III. U/L SH & IH
IV. B/L SH
V. B/L SH & IH
 IH (Infrahyoid)
 SH (Suprahyoid)
 U/L (Unilateral)
 B/L (Bilateral)

6.  It is rare to find lymphangiomas intra - abdominally.
 Most intra-abdominal lymphangiomas occur in
mesentery esp. small bowel mesentery.
 Some cases had been noted in the retroperitoneal,
small bowel, lungs, mediastinum, liver, spleen,
hepatosplenic, bone, pancreas, esophagus, stomach,
colon, biliary system, adrenal, bladder, ovarian.

7. 1. Patient- 15year old female.
2. Chief complaint-
Fever - 3 months
Pain abdomen -2 months
Vomiting and abdominal distension - 15 days
Abd. distension - lower > upper
Not pass flatus and motion - 5 days.
3. Past and family history was not significant.

8.  Hypotension.
 Pallor.
 Edema of hands and feet.

9. Abdominal distension [ lower>upper]
Guarding present.
Rigidity present[lower abdomen]
Bowel sounds absent.

10. • RBI – Hb (6.1gm/dl),
• TLC (20.91x 1000/cumm),
• Decreased serum total proteins , [albumin (2.8 gm/dl)],
• Reversed A:G ratio (1:1.3),
• Hypocalcaemia (7.6 mg/dl).
• XRAY-FPA – MAFL+
• USG – Large collection with septations [numerous] in the
abdomen and pelvic cavity.

11. • Surgical.
• Exploratory Laprotomy was done.
• Per-op. –
Large jejunal mass.
Volvulus of small gut with small gut gangrene.
Proximal 1 feet of jejunum was spared.

12. Fig.1: Showing Large Jejunal Lymphangioma with
Multicystic Appearance.

13. Fig.2: Showing Large Jejunal Lymphangioma with
Volvulus of Small Gut.

14. Fig.3: Showing Large Jejunal Lymphangioma with
Volvulus of Small Gut with Gut Gangrene.

15. Fig.4a: Small Gut Gangrene.

16. Fig.4b: Small Gut Gangrene.

17. Fig.4c: Small Gut Gangrene.

18. • Resection of the mass with gangrenous small
gut and jejuna-ascending colonic anastomosis
done.
• Abdominal drain placed in the pelvic cavity.

19. • Gross-
300cm length small gut.
16x13x8cm mass
Sub mucosal in location.
Extending from mucosa to serosa.
Cut surface of tumor-honey coomb appearance, multiple
tiny cysts.
• Microscopic-
dilated lympho vascular channel, edema, serosa infiltrated
by acute and chronic inflammatory cells, suggestive of
lymphangioma of small intestine.
• Lymphnodes- dilated sinusoid and infiltration by acute
and chronic inflammatory cells.

20. Slide.1: Showing Dilated Submucosal Lymphatic
Spaces.

21. Slide.2: Showing Thin Endothelial Lining with
Inflammatory Cells in the Lumen of Small Gut.

22. • Patient was shifted to surgical ICU.
• Patient was hypotensive and tachycardiac in the post-op. period, managed
by support.
POD-0: 1 PC, 1 FFP, 1 Platelets.
POD-1: 1 PC, 1 FFP, 1 Platelets.
POD-2: Electrolyte & Fluid Imbalance.
POD-3: Electrolyte & Fluid Imbalance – Corrected.
POD-4: Bowel Sounds Present; 1PC.
POD-5: Patient shifted to 3 AB-Ward; 1 PC.
POD-6: Passed Flatus; Ryle’s Tube Removed; Oral Sips of Water.
POD-7: Allowed Oral Liquids.
POD-9: Passed Motion.
POD-10: Drain Removed ; Wean off Support.
POD-11: Allowed Semi Solids & Oral Antibiotics.
POD-12: Oral Solids.
POD-13: Patient Discharged.

23.  15 yrs old female, a case of small bowel
lymphangioma with volvulous and gangrene small
gut managed surgically.
 Post op period- uneventful.
 Discharge on oral antibiotics.

24.  Lymphangiomas are benign lesions of vascular origin that
show lymphatic differentiation.
 They occur in many anatomic locations and may have a
pediatric or adult clinical presentation.
 Most (95%) occur in the neck and axillary regions; the
remaining 5% are located in the mesentery, retroperitoneum,
abdominal viscera, lung, and mediastinum.
 Lymphangiomatosis is a rare disease with multifocal
lymphatic proliferation that typically presents during
childhood and involves multiple parenchymal organs
including the lung, liver, spleen, bone, and skin.
 Because lymphangiomas present across a wide age range of
patient ages and occur in many sites, they are associated with
a broad spectrum of clinical and radiologic manifestations.

25.  An article summarized 107 cases of abdominal
lymphangiomas (58 mesenteric, 11 retroperitoneal, 5
pancreatic, 9 splenic, 8 colonic, 8 small intestinal, 2
renal, 1 hepatic, 1 hepatosplenic, 1 biliary, 1 adrenal, 1
bladder & 1 ovarian) accessioned in the radiologic
pathology archive of the Armed Forces Institute of
pathology over a 22 year period. The purpose of this
study is to describe and illustrate the imaging features of
abdominal lymphangiomas with pathologic correlation.
[Angela D. Levy1, Vito Cantisani & Markku Miettinen]
[American Journal of Roentgenology]

26.  Intramural lymphatic obstruction, disturbed
endothelial permeability, inflammation, congenital
absence of lymphatics, and aging of the bowel wall
have been suggested as causes for the development of
intestinal lymphangiomas.
 Patients with small bowel lymphangiomas have
variable presentations including abdominal pain, an
abdominal mass, abdominal distention, or an acute
abdomen, but most children have acute symptoms.

27.  Volvulus is one of the commonest presenting problems in
children. Children with this may present with an acute
abdomen or intermittent abdominal pain.
 Preoperative imaging will be able to provide more clinical
information, however, the diagnosis can only be conclusively
confirmed after surgery.
 Barium studies show smoothly marginated mural masses that
deform and alter shape when compression is applied [1a, 1b,
1c]. Endoscopic sonography and CT show evidence of cystic
mass in the intestinal wall [Ex-1, 2].
 CT-Scan or MRI or Capsule Endoscopy, providing
information needed for surgical planning.

28. Fig. 1a —Two colonic lymphangiomas in 43-year-old woman
with pancreatic cancer and no colonic symptoms. Single-
contrast enema-enhanced CT scan shows two well-defined oval
filling defects (arrows) in ascending colon.

29. Fig. 1b —Two colonic lymphangiomas in 43-year-old woman
with pancreatic cancer and no colonic symptoms. Photograph
of open resected surgical specimen shows two masses (arrows)
covered with normal mucosa bulging into lumen of ascending
colon. Appendix (a) and ileocecal valve (ic) appear in lower
portion of specimen.

30. Fig. 1c —Two colonic lymphangiomas in 43-year-old woman with
pancreatic cancer and no colonic symptoms. Photomicrograph of
histopathologic specimen shows multiple interconnecting cysts in
submucosa. (H and E, ×4)

31. Example-1: Jejunal lymphangioma in 51-year-old woman with
anemia. Image obtained during enteroclysis shows lobular
filling defect (arrow) in proximal jejunum.
.

32. Example-2: Jejunal lymphangioma in 51-year-old woman with
anemia. Oral and IV contrast–enhanced CT scan shows fluid-
attenuation mural mass (arrow) in proximal jejunum.

33. (a) Capsule endoscopic image shows multiple punctuate white
lesions (circled) in the proximal small bowel.

34. (b) Intraoperative endoscopic image shows markedly thickened
small bowel folds.

35. (c) CT scan shows circumferential low-attenuation wall
thickening in a jejunal segment (circled) causing narrowing of
the lumen and enlargement of the small bowel loop.

36. (d) Intraoperative photograph shows marked distention of a
jejunal loop, a finding that corresponds to the abnormality seen
at CT.

37.  Surgery is the treatment of choice for all small bowel
lymphangiomas, including mesenteric cystic
lymphangiomas.
 Surgeons usually aim for complete removal of the
tumor with surrounding organs of potential invasions,
because there is possibility of recurrence and invasion
to surrounding organs.
 Partial or incomplete tumor removal may also be
associated with complications like infection, fistula,
and hemorrhage.

38. The presented case report concludes:
• Clinical examination & pre op. investigations shows
features of peritonitis.
• Intra op. findings- Jejunal mass with small bowel
volvulus with small gut gangrene.
• Histopathology – small bowel lymphangiomas.
• So, we should consider small bowel lymphangiomas
as a differential diagnosis of peritonitis.

39. THANK YOU